We next investigated the efficacy of TB‐1‐mediated activation of hepatic autophagy for therapy of proximal and distal UCD using the most relevant mouse models for these disorders, the spf‐ash and the AslNeo/Neo mice for OTC and ASL deficiency, respectively (Moscioni et al, 2006; Prieve et al, 2018; Kurtz et al, 2019; Soria et al, 2019). Here, ASL is linked to hyperinsulinemic hypoglycemia, familial, 4.